Localized scleroderma in childhood: A report of 30 cases
Identifieur interne : 002782 ( Istex/Curation ); précédent : 002781; suivant : 002783Localized scleroderma in childhood: A report of 30 cases
Auteurs : Yosef Uziel [Canada] ; Bernice R. Krafchik [Canada] ; Earl D. Silverman [Canada] ; Paul S. Thorner [Canada] ; Ronald M. Laxer [Canada]Source :
- Seminars in Arthritis and Rheumatism [ 0049-0172 ] ; 1994.
English descriptors
- Teeft :
- Achilles tendon, Acta derm venereol, Active phase, Anticentromere antibodies, Antihistone antibodies, Antinuclear antibodies, Arch dermatol, Arthritis rheum, Cerebral calcification, Clinical evaluation, Clinical study, Collagen, Collagen fibers, Convulsive disorders, Coup, Crest syndrome, Degranulated mast cells, Dermatol, Disease activity, Erythrocyte sedimentation rate, Family history, Fibroblast, Fibroblast proliferation, Fibrotic, Frontal lobe, Generalized morphea, Growth factor, Growth failure, Intravenous immunoglobulin, Intravenous methylprednisolone, Laboratory results, Lesion, Limited range, Linear sclcroderma, Linear scleroderma, Localized, Localized cutaneous scleroderma, Localized scleroderma, Many patients, Mast cell activation, Mast cell tryptase, Mast cells, Maxillary antrum, Medical history, Morphea, Mouse model, Muscle atrophy, Neurological symptoms, Nsaid, Pediatric, Pediatric series, Penicillamine, Pigmentary changes, Plastic surgery, Rare disease, Rheum, Rheumatoid factor, Rheumatol, Romberg syndrome, Sabre, Scleroderma, Sclerosis, Skin adnexa, Skin fibrosis, Skin lesion, Skin lesions, Subcutaneous, Subcutaneous tissue, Syndrome, Systemic, Systemic sclerosis, Tiny calcifications.
Abstract
Abstract: Localized scleroderma (LS), a rare disease that occurs primarily in the pediatric age group, differs from systemic sclerosis (SSc) in that it is usually limited to the skin and subcutaneous tissue and is only rarely associated with systemic manifestations. The authors' experience with pediatric LS seen in 30 patients at a tertiary care center was reviewed: 26 had linear scleroderma, 19 on an extremity and 7 on the face; 3 had morphea; and 1 had generalized morphea. Antinuclear antibodies were present in 76% and rheumatoid factor in 39%. Five of 19 patients with linear scleroderma that involved an extremity had growth failure in that limb, and 1 required surgery. Sclerodermatous involvement over a joint resulted in limited range of movement in 6 patients, and 1 required surgery. One of the 30 patients developed SSc and polymyositis. There was difficulty in evaluating disease activity and hence in evaluating treatment. This experience with a large patient population suggests that LS, although usually a self-limiting disease, can result in significant morbidity.
Url:
DOI: 10.1016/0049-0172(94)90028-0
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Silverman</name><affiliation wicri:level="1"><mods:affiliation>Division of Rheumatology, The Hospital for Sick Children, Canada</mods:affiliation><country xml:lang="fr">Canada</country><wicri:regionArea>Division of Rheumatology, The Hospital for Sick Children</wicri:regionArea></affiliation><affiliation><mods:affiliation>3Drs Silverman and Laxer are Associates of the Arthritis Society.</mods:affiliation></affiliation></author><author><name sortKey="Thorner, Paul S" sort="Thorner, Paul S" uniqKey="Thorner P" first="Paul S." last="Thorner">Paul S. Thorner</name><affiliation wicri:level="1"><mods:affiliation>The Hospital for Sick Children, Canada</mods:affiliation><country xml:lang="fr">Canada</country><wicri:regionArea>The Hospital for Sick Children</wicri:regionArea></affiliation><affiliation wicri:level="1"><mods:affiliation>1From the Divisions of Rheumatology and Dermatology, Department of Paediatrics, and the Department of Pathologe, The Hospital for Sick Children: und the Departments of Paediatrics, Immunology, Medicine, and Pathology, the University of Toronto, Toronto, Canada.</mods:affiliation><country xml:lang="fr">Canada</country><wicri:regionArea>1From the Divisions of Rheumatology and Dermatology, Department of Paediatrics, and the Department of Pathologe, The Hospital for Sick Children: und the Departments of Paediatrics, Immunology, Medicine, and Pathology, the University of Toronto, Toronto</wicri:regionArea></affiliation></author><author><name sortKey="Laxer, Ronald M" sort="Laxer, Ronald M" uniqKey="Laxer R" first="Ronald M." last="Laxer">Ronald M. Laxer</name><affiliation wicri:level="1"><mods:affiliation>Address correspondence to Ronald M. Laxer, MD, Division of Rheumatology, The Hospital for Sick Children, 555 University Ave, Toronto, Ontario, Canada M5G 1X8.</mods:affiliation><country>Canada</country><wicri:regionArea>Address correspondence to Ronald M. Laxer, MD, Division of Rheumatology, The Hospital for Sick Children, 555 University Ave, Toronto, Ontario</wicri:regionArea></affiliation><affiliation wicri:level="1"><mods:affiliation>Head, Division of Rheumatology, The Hospital for Sick Children, Canada</mods:affiliation><country xml:lang="fr">Canada</country><wicri:regionArea>Head, Division of Rheumatology, The Hospital for Sick Children</wicri:regionArea></affiliation><affiliation wicri:level="1"><mods:affiliation>1From the Divisions of Rheumatology and Dermatology, Department of Paediatrics, and the Department of Pathologe, The Hospital for Sick Children: und the Departments of Paediatrics, Immunology, Medicine, and Pathology, the University of Toronto, Toronto, Canada.</mods:affiliation><country xml:lang="fr">Canada</country><wicri:regionArea>1From the Divisions of Rheumatology and Dermatology, Department of Paediatrics, and the Department of Pathologe, The Hospital for Sick Children: und the Departments of Paediatrics, Immunology, Medicine, and Pathology, the University of Toronto, Toronto</wicri:regionArea></affiliation></author></analytic><monogr></monogr><series><title level="j">Seminars in Arthritis and Rheumatism</title><title level="j" type="abbrev">YSARH</title><idno type="ISSN">0049-0172</idno><imprint><publisher>ELSEVIER</publisher><date type="published" when="1994">1994</date><biblScope unit="volume">23</biblScope><biblScope unit="issue">5</biblScope><biblScope unit="page" from="328">328</biblScope><biblScope unit="page" to="340">340</biblScope></imprint><idno type="ISSN">0049-0172</idno></series></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0049-0172</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="Teeft" xml:lang="en"><term>Achilles tendon</term><term>Acta derm venereol</term><term>Active phase</term><term>Anticentromere antibodies</term><term>Antihistone antibodies</term><term>Antinuclear antibodies</term><term>Arch dermatol</term><term>Arthritis rheum</term><term>Cerebral calcification</term><term>Clinical evaluation</term><term>Clinical study</term><term>Collagen</term><term>Collagen fibers</term><term>Convulsive disorders</term><term>Coup</term><term>Crest syndrome</term><term>Degranulated mast cells</term><term>Dermatol</term><term>Disease activity</term><term>Erythrocyte sedimentation rate</term><term>Family history</term><term>Fibroblast</term><term>Fibroblast proliferation</term><term>Fibrotic</term><term>Frontal lobe</term><term>Generalized morphea</term><term>Growth factor</term><term>Growth failure</term><term>Intravenous immunoglobulin</term><term>Intravenous methylprednisolone</term><term>Laboratory results</term><term>Lesion</term><term>Limited range</term><term>Linear sclcroderma</term><term>Linear scleroderma</term><term>Localized</term><term>Localized cutaneous scleroderma</term><term>Localized scleroderma</term><term>Many patients</term><term>Mast cell activation</term><term>Mast cell tryptase</term><term>Mast cells</term><term>Maxillary antrum</term><term>Medical history</term><term>Morphea</term><term>Mouse model</term><term>Muscle atrophy</term><term>Neurological symptoms</term><term>Nsaid</term><term>Pediatric</term><term>Pediatric series</term><term>Penicillamine</term><term>Pigmentary changes</term><term>Plastic surgery</term><term>Rare disease</term><term>Rheum</term><term>Rheumatoid factor</term><term>Rheumatol</term><term>Romberg syndrome</term><term>Sabre</term><term>Scleroderma</term><term>Sclerosis</term><term>Skin adnexa</term><term>Skin fibrosis</term><term>Skin lesion</term><term>Skin lesions</term><term>Subcutaneous</term><term>Subcutaneous tissue</term><term>Syndrome</term><term>Systemic</term><term>Systemic sclerosis</term><term>Tiny calcifications</term></keywords></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Abstract: Localized scleroderma (LS), a rare disease that occurs primarily in the pediatric age group, differs from systemic sclerosis (SSc) in that it is usually limited to the skin and subcutaneous tissue and is only rarely associated with systemic manifestations. The authors' experience with pediatric LS seen in 30 patients at a tertiary care center was reviewed: 26 had linear scleroderma, 19 on an extremity and 7 on the face; 3 had morphea; and 1 had generalized morphea. Antinuclear antibodies were present in 76% and rheumatoid factor in 39%. Five of 19 patients with linear scleroderma that involved an extremity had growth failure in that limb, and 1 required surgery. Sclerodermatous involvement over a joint resulted in limited range of movement in 6 patients, and 1 required surgery. One of the 30 patients developed SSc and polymyositis. There was difficulty in evaluating disease activity and hence in evaluating treatment. This experience with a large patient population suggests that LS, although usually a self-limiting disease, can result in significant morbidity.</div></front></TEI></record>Pour manipuler ce document sous Unix (Dilib)
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